Extrahepatic biliary tract injury: operative management plan

Trauma to the extrahepatic biliary tract is rare but, if overlooked or improperly managed, may be associated with significant morbidity. The following is our most recent 6-year experience with this unusual injury. Among 949 patients undergoing laparotomy for acute trauma, there were 32 (3%) injuries to the gallbladder and five (0.5%) to the common bile duct. All patients with gallbladder injury underwent abdominal exploration because of associated trauma. Cholecystectomy was preferred for 24 (96%) of the 25 with penetrating wounds and three (43%) of the seven with blunt trauma. Nine (28%) patients developed postoperative complications but none were related to the biliary injury. All common bile duct injuries (CBD) were penetrating. Associated intra-abdominal trauma was always present. Partial lacerations were closed primarily over a T-tube. An intrapancreatic CBD lesion was managed by sphincteroplasty and stented with a T-tube. The one patient with complete transection underwent choledochoenterostomy. Postoperative complications were common (50%) but none were related to the biliary injury.     

Extrahepatic biliary atresia and caudal regression syndrome in an infant of a diabetic mother

Although most cases of extrahepatic biliary atresia are thought to result from perinatal obliterating inflammation, some are associated with a faulty morphogenesis. The authors report on a baby girl of a mother with ill-managed insulin-dependent diabetes mellitus. The baby presented with sacro-coccygeal agenesis, clubfoot, and ano-urinary incontinence. In addition, there was polysplenia, no inferior vena cava, and the portal vein was grossly distorted. Progressive conjugated hyperbilirubinemia prompted liver biopsy at 4 weeks of age, showing intracanalicular cholestasis without fibrosis. The diagnosis of extrahepatic biliary atresia was confirmed during laparotomy performed for hepatoportojejunostomy. Some cases of extrahepatic biliary atresia might be part of a spectrum of malformations associated with maternal diabetes mellitus.     

Surgical management for intractable cholangitis in biliary atresia

Background/Purpose: The efficacy of antireflux surgical procedures involving the Roux-en-Y jejunal limb for cholangitis was evaluated retrospectively in patients with biliary atresia (BA). Methods: From July 1993 to December 2001, 41 patients with BA underwent hepatic portojejunostomy with Roux-en-Y reconstruction. Of these patients, 11 had intractable cholangitis that was treated by creation of a value with or without lengthening of the Roux-en-Y limb. Results: Among the 11 patients, the first episode of cholangitis occurred within 6 months after portojejunostomy in 10 patients and at the age of 4 years in one patient. Cholangitis developed at various intervals from once every week to once every 2 months requiring hospitalization each time. All patients underwent valve creation at 2 months to 5 years postoperatively, whereas 2 had an additional lengthening of the limb. Cholangitis resolved completely after surgery in all cases. Two patients underwent liver transplantation, and the third patient died of an unrelated cause. The 8 survivors with native livers are doing well after 1 to 8 years of follow-up. Conclusion: Early surgical intervention could control intractable cholangitis in all patients, both delaying the time of liver transplantation and improving the quality of life.     

Technique and results of operative management of biliary atresia

Intrahepatic bile ducts are patent in the liver of patients with biliary atresia in early infancy. Small bile ducts at the porta hepatis continuing to the intrahepatic ducts usually terminate in fibrous tissue replacing the hepatic radicles, which does not extend to the common hepatic duct. Therefore, the level of transection of the fibrous tissue at the porta hepatis is of prime importance for successful hepatic portoenterostomy. Transection should be done at the level of the posterior surface of the portal vein. The overall cure rate of surgery for biliary atresia was 30%. In our recent series, however, the results have markedly improved and the cure rate has been increased to 55%. There are 47 patients living without jaundice, 22 of them for more than 5 years. The longest survivors are 23 years old. All the longterm survivors are leading a normal life for their age, although 6 of them have some physical or mental handicap. Early operation, adequate surgical technique, and prevention of postoperative cholangitis are requisites for successful hepatic portoenterostomy.

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Résumé Chez les nouveaux-nés atteints d'atrésie biliaire, les canaux biliaires intra-hépatiques sont perméables. Dans le hile hépatique, ces canaux intrahépatiques se terminent par de fins canalicules enserrés dans du tissu fibreux, qui ne se poursuivent pas jusqu'au canal hépatique commun. Le niveau de section du tissu fibreux dans le hile hépatique est donc très important pour le succès de l'hépaticoentérostomie. Cette section doit être faite au niveau de la face postérieure de la veine porte. La chirurgie pour atrésie biliaire donnait 30% de guérisons. Dans notre récente série, les résultats ont été améliorés jusqu'à 55% de guérisons. Nous avons 47 malades qui vivent sans ictère, dont 24 depuis plus de 5 ans. Les plus longues survies atteignent 23 ans. Tous ont une vie normale pour leur âge, malgré le fait que 7 d'entre eux présentaient certains handicaps physiques ou mentaux. Le succès de l'hépaticoentérostomie dépend de la précocité de l'intervention, de la qualité de la technique opératoire et de la prévention de l'angiocholite postopératoire.

     

Systematic review: The quality of life of patients with biliary atresia

BackgroundThe quality of life of patients with Biliary Atresia (BA) have not been systematically examined. The goal of this meta-analysis is to determine patients' postoperative health-related Quality of life (HrQoL) with native or transplanted livers.MethodsFrom 2000 to August 2021, a literature-based search for relevant cohorts was conducted using Pubmed/Medline, the Cochrane Library, and Embase. Original research on BA, Hepatoportoenterostomy (HPE), portoenterostomy, Kasai, Liver transplantation and HrQoL was included. Using RevMan, a forest plot analysis of HrQoL after surgical treatment after BA was calculated (version 5.4). Using MetaXL, a pooled prevalence for cholangitis, secondary liver transplantation, or related malformations was computed (version 5.3).ResultsNine studies compared individuals with BA to an age-matched healthy control group. 4/9 (n = 352) of these studies found poorer scores for BA patients, while 5/9 (n = 81) found equivalent health status. Factors associated with HrQoL: older age at the time of the survey was linked to greater HrQoL; whereas females, higher total bilirubin and the amount of immunosuppressive medicines were associated with lower HrQoL in BA patients.ConclusionThe current study emphasises the critical need to improve the many parameters influencing HrQoL in BA patients, as well as the methods utilized to assess those factors. This includes immunosuppression, withdrawal from polydrug regimes and recognizing the differences in disease burden between males and females.Type of studySystematic review.Level of evidenceLevel III.     

Idiopathic thrombocytopenic purpura complicated with biliary atresia: a rare occurrence and literature review

The association of biliary atresia (BA) and idiopathic thrombocytopenic purpura (ITP) is extremely rare, with only 2 cases being reported in the literature. This report describes the very rare case of a 1-year-old boy with BA complicated with ITP after cholangitis and the successful steroid treatment of ITP. We review the literature on this type of relationship between BA and ITP and discuss the clinical features of this complication. Furthermore, the possible cause of the onset of ITP complicated with BA was explored in this report.     

Extrahepatic biliary obstruction by lymphoma

Extrinsic compression of the common bile duct by enlarged lymph nodes is an unusual cause of obstructive jaundice. We describe two patients with such a manifestation of lymphoma and the use of endoscopic retrograde cholangiopancreatography in distinguishing this rare complication from other causes of jaundice in lymphoma. Patients may have rapid resolution of both symptoms and biochemical abnormalities by nonoperative biliary bypass combined with chemotherapy and/or radiotherapy. Lymphomatous obstruction should be considered a cause of potentially treatable jaundice in patients with known lymphoma; endoscopic retrograde cholangiopancreatography can be valuable as a diagnostic and therapeutic tool.     

Congenital talipes equinovarus: a review of current management

Talipes equinovarus is one of the more common congenital abnormalities affecting the lower limb and can be challenging to manage. This review provides a comprehensive update on idiopathic congenital talipes equinovarus with emphasis on the initial treatment. Current management is moving away from operative towards a more conservative treatment using the Ponseti regime. The long-term results of surgical correction and the recent results of conservative treatment will be discussed.     

肝外胆道变异与医源性胆管损伤

医源性胆道损伤(iatrogenic bile duct injury)是每一位胆道外科医生职业生涯中难以回避的困扰,发生率约0.5%.究其原因与肝外胆道的解剖变异较为复杂密切相关,胆囊和肝外胆道的解剖变异发生率可高达50%.本文就肝外胆道变异与胆管损伤的关系加以探讨.     

Mother-to-daughter occurrence of biliary atresia: a case report

We herein report a case of mother-to-daughter occurrence of biliary atresia (BA). The mother underwent a Kasai hepatic portoenterostomy at the age of 64 days with a diagnosis of type III BA. Her postoperative course was uneventful, with a good bile discharge, and she became pregnant at the age of 20 years. At 21 weeks of gestational age, fetal ultrasonographic examination of the baby revealed a cystic mass at the hepatic hilum. The baby was born via vaginal delivery at 41 weeks of gestation with a birth weight of 3269 g. Because of an increasing direct bilirubin level and the absence of bile excretion, a laparotomy was performed at the age of 29 days. Intraoperative cholangiography revealed the diagnosis of BA I-cyst, and a hepaticojejunostomy was performed with a good bile discharge. To our knowledge, these patients are the first occurrence of BA in a transmission pattern of mother to daughter.     

胆道闭锁诊治现状与展望

胆道闭锁是新生儿黄疸常见病因之一。其发病率约1/ 10 0 0 0 ,多见于母乳喂养儿,出生时体重正常,女性多于男性。长期以来,儿外科工作者对此病进行了广泛深入的研究,迄今为止,发病原因尚不明确。胆管闭锁发生的时间可以是胎儿期、或出生后,因此,目前只提“胆道闭锁”,而不是冠以     

The surgical management of the unusual forms of biliary atresia

Of 50 infants undergoing laparotomy for biliary atresia during the last 9 yr at the Kobe Children's Hospital, 9 had variant forms, including (A) a gallbladder with a demonstrable connection with the intrahepatic biliary system, (B) a gallbladder with a connection with the extrahepatic biliary system and duodenum, and (C) a grossly visible subhepatic cyst. Three patients with form A underwent cholecystoduodenostomy. All these patients died of cholangitis and hepatic failure within 2 mo after operation. Two patients with form B were successfully treated by hepatic portocholecystostomy without any episodes of cholangitis. Two of the patients with form C, occurring in 1970, underwent an anastomosis between the subhepatic cyst and the duodenum. There was no bile excretion in these patients. The other two patients were treated subsequently by hepatic portoenterostomy with complete excision of the subhepatic cyst and excellent bile excretion was obtained. Infants with forms A and C should be treated by hepatic portoenterostomy instead of a direct anastomosis between the dilated portion of the extrahepatic biliary system and the alimentary tract. For patients with form B, hepatic portocholecystostomy seems to be the most suitable procedure relative to the prevention of postoperative cholangitis, which is the most serious complications in infants obtaining successful bile excretion.     

Combined pure esophageal atresia, duodenal atresia, biliary atresia, and pancreatic ductal atresia: prenatal diagnostic features and review of the literature

The authors recently managed a case of combined pure esophageal, duodenal, biliary, and pancreatic ductal atresia in a fetus and newborn with Trisomy 21. The authors present a case report and review of the literature, emphasizing the prenatal radiographic features of the combined lesions and the high incidence of associated anomalies including Down's syndrome. Prenatal suspicion of these anomalies warrants karyotype analysis as well as careful pre- and postnatal screening for other anomalies.     

Orthotopic liver transplantation for biliary atresia. Evolution of management

Forty-five patients with biliary atresia were accepted for orthotopic liver transplantation. Nine patients died awaiting transplantation, and 36 underwent transplantation. A portoenterostomy had been performed in 28 of these 36 patients, and its presence did not significantly affect the intraoperative blood loss (5.6 vs 4.1 blood volumes), the need for retransplantation (21% vs 12%), biliary complications (21% vs 12%), postoperative infections (36% vs 25%), or survival (82% vs 63%). These results indicate that early portoenterostomy is appropriate early therapy for biliary atresia; however, prompt referral to a liver transplant center for evaluation at the first sign of cholestasis is needed to attain optimal results for transplantation. Revisions of the portoenterostomy prior to transplantation did not improve the longevity of the procedure but did substantially increase complications and death after orthotopic liver transplantation.